pathology: Inflammatoryinfiltrate and fibrosis of unknown cause
Aetiology: Commonestcause of interstitial lung disease (ILD)
Alsoknown as cryptogenic fibrosing alveolitis (CFA)
Symptoms: Dry cough and exertional dyspnoea
Signs: Bilateralfine end-inspiratory crackles, clubbing
Investigations: Clinical,exclude other diagnoses and causes
Treatment: Steroids,azathioprine, cyclophosphamide, transplant
Complications: 10%develop lung tumour
Prognosis: 50%at 5 years
